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Desmoplastic Trichoepithelioma: An Overview

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Desmoplastic trichoepithelioma (DTE) is a hair follicle-derived benign cutaneous condition. Read the article to know more.

Medically reviewed by

Dr. S. Renjana

Published At October 16, 2023
Reviewed AtJanuary 30, 2024

Desmoplastic trichoepitheliomas (DTE) are benign cutaneous neoplasms that arise from the hair follicle and preferentially affect the face. Desmoplastic trichoepitheliomas (DTE) is a relatively rare benign cutaneous adnexal tumor. This form of tumor is distinguished by rapid development and robust histologic and immunohistochemical characteristics that may be mistaken for other types of skin cancer. The preferred therapy is local excision. However, when the therapeutic features of desmoplastic trichoepitheliomas (DTE) are unique, a observe and see technique might be applied as a management practice.

What Is Desmoplastic Trichoepithelioma?

Desmoplastic trichoepithelioma (DTE) is a rare tumor due to its non-neoplastic (cancerous) character, absence of ulceration, histological appearance, superficial invasion, and early onset. Desmoplastic trichoepithelioma (DTE), like other types of skin cancer, grows slowly at first and later becomes a stable lesion.

How Is Desmoplastic Trichoepithelioma Classified?

Desmoplastic trichoepithelioma (DTE) is classified into three types:

  1. Solitary trichoepithelioma.

  2. Numerous trichoepithelioma.

  3. Desmoplastic trichoepithelioma.

What Is the Incidence of Desmoplastic Trichoepithelioma?

Desmoplastic trichoepithelioma (DTE) is a rare benign appendageal skin cancer that occurs in two out of every 10,000 people and accounts for less than 1 % of all cutaneous malignancies. Desmoplastic trichoepithelioma (DTE) has a bimodal age distribution and usually affects young children or adults. Because of its distinct clinical and histological characteristics, it is a particular form of trichoepithelioma.

What Is the Occurrence of Desmoplastic Trichoepithelioma?

It generally manifests as a single lesion, although multiple lesions have been recorded in rare situations. Most desmoplastic trichoepithelioma (DTE) manifests as a white to yellowish annular nodule or papule with a central depression. Female patients are the most commonly afflicted, and the most common place is on the face or cheeks. It usually has no indications or symptoms and appears as a single, indurated, annular, and centrally depressed papule or plaque.

What Are the Symptoms of Desmoplastic Trichoepithelioma (DTE)?

According to research, there is a hereditary chance of developing trichoepithelioma; periodic monitoring might aid in early detection. There are usually no symptoms associated with the lesion, although it may progressively grow in size and shape over time. Sun-exposed parts are the most frequently impacted, particularly face regions such as the cheeks, chin, and forehead. Less often, malignancies can be seen in the neck, upper trunk, and scalp. Desmoplastic trichoepithelioma (DTE) has a consistent development pattern and can reach a diameter of one centimeter. Multiple lesions are uncommon. Although rare, trichoepithelioma can progress to trichoblastic carcinoma or basal cell carcinoma (BCC).

How Is Desmoplastic Trichoepithelioma (DTE) Diagnosed?

Desmoplastic trichoepithelioma (DTE) diagnosis can be difficult, especially when the tumor mimics other benign and malignant tumors. Histological evidence, in conjunction with clinical symptoms, may, on the other hand, help obtain a definitive diagnosis of sure of these lesions.

  • Skin Cancer Screenings - Desmoplastic trichoepithelioma (DTE) may be detected via standard skin cancer screenings and can be present on the skin for many years without causing any changes or symptoms. Skin cancer screening as part of routine physical examinations, follow-up and biopsy, and ongoing monitoring are all critical.

  • Full-Thickness Skin Biopsy- Desmoplastic trichoepithelioma (DTE) is best diagnosed with a full-thickness skin biopsy. Because basal cell carcinoma (BCC) and microcystic adnexal carcinoma might seem similar, small partial samples can lead to confusion. In questionable circumstances, resampling or re-excision may be required for a precise diagnosis or total eradication. Although clinical diagnosis is sometimes challenging and necessitates histopathological expertise, a skin biopsy may be necessary based on the accurate location of the lesion, change in shape, or tumor development. In addition, the pathology report determines whether the lesion should be monitored or surgically removed. As a result, a preliminary skin biopsy is vital for making the correct diagnosis and choosing the best therapy choices for a patient.

What Is the Treatment for Desmoplastic Trichoepithelioma (DTE)?

Surgical Excision -

Surgical excision is the preferred therapy for single lesions on various body areas. Mohs microscopic surgery is advised for facial lesions to acquire clean surgical margins. When cancer cells and deep tissue invasion are found, complete excision should be undertaken to rule out malignancy when several lesions need to be treated.

Mohs Microscopic Surgery -

Mohs microscopic surgery is preferable for lesions on the face. Desmoplastic trichoepithelioma (DTE) can be challenging to identify from other cutaneous adnexal neoplasms. There is a significant distinction between desmoplastic trichoepithelioma (DTE) and other cutaneous malignancies. Additional research is needed to develop the definitive diagnostic clinical aspects of cutaneous adnexal tumors.

What Is the Differential Diagnosis for Desmoplastic Trichoepithelioma (DTE)?

The differential diagnosis for desmoplastic trichoepithelioma (DTE) is as follows:

  • Morphea basal cell carcinoma (MBCC).

  • Conventional trichoepithelioma.

  • Syringoma.

  • Microcystic adnexal carcinoma (MAC).

  • Other malignancies.

How Can Desmoplastic Trichoepitheliomas (DTE) And Basal Cell Carcinoma Be Distinguished?

Five unique clinicopathological features may distinguish desmoplastic trichoepitheliomas (DTE) and basal cell carcinoma:

  • Annular lesions.

  • Horn-cysts.

  • Epidermal hyperplasia.

  • Keratin granulomas.

  • Calcification.

  • Large aggregates of tumor cells, common in basal cell carcinoma, are seldom seen in desmoplastic trichoepitheliomas (DTE).

How Can Desmoplastic Trichoepitheliomas (DTE) And Microcystic Adnexal Carcinoma Be Distinguished?

Microcystic adnexal carcinoma is an uncommon adnexal neoplasm that often affects the head and neck area, especially the central face. In contrast, desmoplastic trichoepitheliomas (DTE) are benign tumors with indolent activity. Unfortunately, MAC can be aggressive, causing significant local damage and probable metastasis. Although MAC is primarily recognized as a distinct clinicopathological entity, misunderstanding benign adnexal tumors, notably DTE, is still possible. In up to 30 % of patients, superficial biopsies misdiagnose MAC as squamous cell carcinoma, syringoma, or DTE.

Conclusion

Desmoplastic trichoepitheliomas (DTE), also called sclerosing epithelial hamartomas, are hair follicle-derived benign cutaneous neoplasms. Desmoplastic trichoepitheliomas (DTE) account for fewer than one percent of all cutaneous neoplasms and are associated with one entity in the broad spectrum of benign follicular differentiated appendageal skin tumors. A hereditary propensity to develop trichoepithelioma exists. Desmoplastic trichoepithelioma is linked to chromosomal alterations on 9p21 and 16q12-q13. Multiple familial trichoepitheliomas are caused by autosomal-dominant conditions and are distinguished by positive family history, histological features, and several papules or nodules.

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Dr. S. Renjana
Dr. S. Renjana

Dermatology

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